Adenocarcinoma of the ampulla of Vater is an uncommon malignancy. Jaundice is the most common presentation of ampullary cancer, and about 70% of patients present with jaundice. Other common symptoms include abdominal pain and weight loss. Only 3-5 % of patients with ampullary cancer are associated with acute pancreatitis. About one-third of patients with ampullary cancer present anemia. We report a case of ampullary cancer, presenting with severe anemia and acute pancreatitis but not jaundice. Abdominal ultrasonography and abdominal computed tomography revealed dilatation of the common bile duct, intra-hepatic duct, and pancreatic duct. Endoscopic retrograde cholangiopancreatogram showed a tumor of the ampulla of Vater, and biopsy proved adenocarcinoma of the ampulla of Vater. The patient underwent Whipple's resection, and adjuvant chemotherapy and radiotherapy. She died 10 months after operation because of cancer recurrence and metastasis.